scholarly journals Randall-type monoclonal immunoglobulin deposition disease: description of cardiac involvement

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A Ramonatxo ◽  
R Garcia ◽  
F Joly ◽  
B Degand ◽  
N Bidegain ◽  
...  
Keyword(s):  
Heart Disease ◽  
Mayo Clinic ◽  
Cardiac Involvement ◽  
Global Longitudinal Strain ◽  
Left Ventricular ◽  
Basement Membranes ◽  
Al Amyloidosis ◽  
Monoclonal Immunoglobulin ◽  
Amyloid Heart Disease ◽  
Heavy Chains

Abstract Background Randall-type monoclonal immunoglobulin disease (MIDD) is a rare complication of a monoclonal plasma cell clone. MIDD differs from AL amyloidosis by the presence of Congo red negative non-organized immunoglobulin (Ig) deposits, most commonly light chains (LCDD) along basement membranes and sometimes heavy chains (HCDD) or light and heavy chains (LHCDD). As AL amyloidosis MIDD is a multi-systemic disease, and affects the heart. To date no study has focused on the clinical characteristics of heart disease in MIDD. Purpose The aim of this study was to describe the cardiologic features of patients with biopsy-proven MIDD and suspected cardiac involvement. Methods This multi-center, nation-wide retrospective study extracted from the database of the French reference center for AL amyloidosis and other Ig deposition diseases between 2012 to 2019. Diagnosis of cardiac involvement was assessed according to the International Society of Amyloidosis criteria for amyloid heart disease, as follows: left ventricular hypertrophy with a diastolic septum thickness ≥12mm, NTproBNP serum level ≥332 ng / L, histological evidence on cardiac or extra cardiac biopsy of typical linear non-organized Ig deposits along basement membranes. Severity was defined according to the Mayo Clinic classification for AL amyloidosis. Results Among 20 patients included (mean age was 70±9 years), 11 (55%) were males; 13 (65%) were LCDD, 3 (15%) HCDD and 4 (20%) LHCDD. At diagnosis, 19 (95%) had a history of hypertension, 3 (16%) had atrial fibrillation, 3 (15%) had NYHA grade 3 or 4 dyspnea. Mayo Clinic score was stage 3a in 4 patients (20%) and stage 3b in 6 patients (30%). The most frequent ECG changes were microvoltage (40%) and pseudo Q wave (40%); 64% of patients had altered sinus variability on 24-hour Holter monitoring, one patient had a high-grade conduction disorder and another had ventricular tachycardia. On echocardiography, all showed diastolic dysfunction; mean diastolic septum thickness was 13.5mm; only one patient had LVEF impairment but 38% had global longitudinal strain impairment. 10 patients had cardiac MRI, none showed contrast enhancement after gadolinium injection. After median follow-up of 28 months, 4 patients were hospitalized for heart failure, including 2 with cardiogenic shock. Seven (35%) patients died within a median of 10 months from diagnosis. Among patients with Mayo clinic stage 3 (a or b), 67% died within a median of 8 months from the diagnosis. Conclusions To our knowledge, we present the first case series dedicated to the description of cardiac parameters in MIDD patients with cardiac involvement. Except for MRI appearance of cardiac infiltration, these patients showed features close to that of AL amyloid heart disease. Overall prognosis appears seemingly poor in MIDD patients with Mayo Clinic stage 3 cardiac disease. Funding Acknowledgement Type of funding source: None

Diagnostic score of cardiac involvement in AL amyloidosis

2019 ◽  
Vol 21 (5) ◽  
pp. 542-548 ◽  
Author(s):  
Martin Nicol ◽  
Mathilde Baudet ◽  
Stephanie Brun ◽  
Stephanie Harel ◽  
Bruno Royer ◽  
...  
Keyword(s):  
Cardiac Involvement ◽  
Troponin T ◽  
Characteristic Curve ◽  
Global Longitudinal Strain ◽  
Left Ventricular ◽  
Cardiac Biomarkers ◽  
Blood Levels ◽  
Al Amyloidosis ◽  
Derivation Cohort ◽  
Diagnostic Score

Abstract Aims  Early diagnosis of cardiac involvement is a key issue in the management of AL amyloidosis. Our objective was to establish a diagnostic score of cardiac involvement in AL amyloidosis and to compare it with the current consensus criteria [i.e. left ventricular hypertrophy >12 mm and N-terminal pro b-type natriuretic peptide (NT-proBNP) >332 ng/L]. Methods and results  We carried out a prospective and multicenter study on AL amyloidosis patients who underwent cardiac evaluation including clinical examination, electrocardiography (ECG), cardiac biomarkers, transthoracic echocardiography (TTE), and cardiac magnetic resonance imaging (CMR). Cardiac involvement was based on CMR and/or endomyocardial biopsy. In a derivation cohort of 114 patients (82 with cardiac involvement), the highest diagnostic accuracy was observed with NT-proBNP and troponin blood levels, TTE-derived global longitudinal strain (LS), and apical to basal LS gradient. By using multivariate analysis, we established a diagnostic score including global LS ≥−17% (1 point), apical/(basal + median) LS ≥0.90 (1 point), and troponin T >35 ng/L (1 point). A score >1 was associated with sensitivity of 94% and specificity of 97%, an area under the curve of 0.98 [95% confidence interval (CI) 0.93–0.99] as well as a net reclassification index of 0.39 (95% CI 0.28–0.46) when compared with consensus criteria. In a validation cohort of 73 AL amyloidosis patients, the area under the receiver operating characteristic curve of the diagnostic score was 0.97 (95% CI 0.90–0.99). Conclusion  Combining T troponin blood levels and two echo-derived strain parameters leads to very high accuracy for diagnosing cardiac involvement in AL amyloid patients.

Weekly Combination Chemotherapy with Cyclophosphamide, Bortezomib and Dexamethasone (CyBorD) for Newly Diagnosed Patients with Advanced Cardiac Disease Due to Systemic AL Amyloidosis

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 5139-5139
Author(s):  
Furha I. Cossor ◽  
Adam Boruchov ◽  
Michael Danso ◽  
Michael Edward Lee ◽  
Ayan Patel ◽  
...  
Keyword(s):  
Standard Therapy ◽  
Cardiac Involvement ◽  
Troponin I ◽  
Single Agent ◽  
Left Ventricular ◽  
Stage Iii ◽  
Al Amyloidosis ◽  
Cell Transplant ◽  
Newly Diagnosed ◽  
Hematologic Response

Abstract Abstract 5139 The treatment of systemic AL amyloidosis (AL) with advanced cardiac involvement (cardiac biomarker stage III) at diagnosis remains challenging and unsatisfactory. Median survival with melphalan and dexamethasone (MDex) in stage III patients is about 10 months from diagnosis with one quarter of patients dying within 3 months of starting therapy (BJH 2008;143:369; Blood 2010;116:522). Bortezomib has been shown to be the most active single agent in the treatment of AL (Blood 2011;118:865) and, when incorporated into CyBorD, clinical studies have shown a > 90% hematologic response rate in both myeloma and in AL (Blood 2010;115:3416; Leukemia 2009;23:1337; Amyloid 2010;17(S1):171a). In addition, bortezomib has been shown to have limited cardiac toxicity and no arrhythmogenicity in patients with AL and cardiac involvement (QJM 2011 published online July 13, 2011). Based on these data we have used CyBorD as initial standard therapy in patients with newly diagnosed systemic AL amyloidosis with advanced (stage III) cardiac involvement who are ineligible for stem cell transplant or clinical trials. We now retrospectively report the outcomes in the 11 consecutive stage III patients who have been treated thus far with CyBorD as initial standard therapy (6F, 5M). Patients were a median of 57 years old (range, 44–74) with median brain natriuretic peptide (BNP) of 709 (145–1490), troponin I of 0.29 (0.11–0.77), involved free light chains of 322mg/L (101–4325) and marrow plasma cells of 20% (4–41%). Median left ventricular (LV) ejection fraction and LV wall thickness (average of IVSd+LVPWd) by echocardiogram were 52% (35–70%) and 1.7cm (1.2–2.1) respectively. Patients received CyBorD on a 35-day cycle on days 1, 8, 15 and 22 with cyclophosphamide (300mg/m2) and dexamethasone (20 or 40mg flat dose) given orally or IV and bortezomib (1.3mg/m2) given IV or subcutaneously. Routine prophylactics included acyclovir, fluconazole and omeprazole. Patients have received a median of 4 cycles (1–8). Of the 11 patients, 2 died suddenly at 1 and 5 months, and 9 are alive with a median follow up of 8 months (3–15). Three patients experienced worsening congestive heart failure requiring hospitalization, medication adjustments and in 1 case pericardiocentesis. The gastrointestinal (GI) side-effects of bloating and constipation were common and usually manageable although 1 patient stopped therapy after 2 cycles because of lower GI bleeding. Of 10 patients evaluable for hematologic response, 8 responded (1 CR, 4 VGPR, 3 PR) (response criteria from Blood 2010;116:586a) and 2 had no response. Median time to response was 1 cycle (1–3). Thus far, there have been 4 patients (2 VGPR, 2 PR) who within months of starting therapy have had BNP reductions of 40% to 76% of baseline (median BNP reduction of 434pg/ml (282–712)). In conclusion, these retrospective data are encouraging and hopefully will spur the development of phase II and III trials in newly diagnosed patients with advanced cardiac involvement due to AL. Disclosures: Off Label Use: Pentostatin and Extracorporeal photopheresis are not FDA approved for conditioning prior to allogeneic transplant.

A Prospective Study of Treatment Outcomes in 179 Patients with Advanced Cardiac Stage STAGE IIIb Amyloidosis

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 5357-5357
Author(s):  
Belen Sevillano ◽  
Ashutosh Wechalekar ◽  
Darren Foard ◽  
Carol Whelan ◽  
Marianna Fontana ◽  
...  
Keyword(s):  
Partial Response ◽  
Prospective Study ◽  
Treatment Outcomes ◽  
Cardiac Involvement ◽  
Left Ventricular ◽  
Stage Iiib ◽  
Stage Iii ◽  
Al Amyloidosis ◽  
Hematological Response ◽  
A Prospective Study

Abstract A PROSPECTIVE STUDY OF TREATMENT OUTCOMES IN 179 PATIENTS WITH ADVANCED CARDIAC STAGE IIIB AMYLOIDOSIS Authors: Belen Sevillano, Darren Foard, Carol Whelan, Mariana Fontana, Critina Quarta, Shameem Mahmood, Helen Lachmann, Julian Gillmore, Philip Hawkins and Ashutosh Wechalekar INTRODUCTION The prognosis of systemic light chain amyloidosis is determined by extent of cardiac involvement. The Mayo cardiac staging system (Dispenzieri et al JCO 2004) is widely used for prognosis and we defined a particularly poor prognostic subgroup within Mayo stage III patients (Wechalekar et al Blood, 121(17), 2013) characterized by NT-proBNP >8500 ng/L with a median survival of 4 months-stage IIIb disease. All such patients are excluded from clinical trials and treatment outcomes of this group are not known. We report the treatment outcomes of a cohort of stage IIIb cardiac AL patients prospectively followed up in the ALChemy study PATIENTS AND METHODS. All patients from the ALChemy study (a prospective observational study of all patients with AL amyloidosis undergoing chemotherapy) at the UK National Amyloidosis Centre (identified from first 1000 patients recruited into the study from 2009 to Jan 2015) with Mayo stage IIIb cardiac AL (defined as NT-proBNP >8500 ng/L and cardiac troponin T >0.035 μg/L) were included. All were treated according to nationally agreed protocols. Organ involvement and hematologic/amyloidotic organ responses were assessed according to 2010 amyloidosis consensus criteria. The primary outcome measure was overall survival (OS) and impact of hematological response on survival. Statistical analysis was undertaken using SPSS software package. Survival was assessed by the Kaplan-Meier method and compared by log-rank test. RESULTS A total of 179 patients were included. The median age was 65 yrs, 77 (43 %) were female and 102 (57 %) male. All patients had cardiac involvement, renal involvement was seen in 131 (73.2 %) and liver involvement in 28 (15.6 %). The median NT-proBNP was 19056 ng/L (range 8500 - 70084 ng/L). The median left ventricular (LV) wall thickness was 14.2 mm (range 11-22 mm) and median ejection fraction (EF) was 48.7 % (23-75 %). 34 % had dyspnea ≥ NYHA grade 3 and 16.8 % had ECOG performance status ≥3. Only 68 (38 %) patients managed a 6 min walk test and median distance was 130.5 m. 30 (17 %) patients died prior to treatment initiation. Initial treatment regimens were: Bortezomib combinations - 48 % (87); Thalidomide or Lenalidomide combinations in 28 % (51), alkylators based regimens - 5 % (9) and rituximab based in 1 %. The hematological best responses on an intention to treat basis were: complete response (CR) - 35w (20%), very good partial response (VGPR) 25 (13%), partial response (PR) 32 (18%) and no response (NR) 87 (47%) (NR included patients who died before treatment). The median OS for the cohort was 6 months (mo). Univariate and ROC analysis identified LVEF >55%, dFLC <400 mg/L and SBP >110 mm of Hg predictors of OS. The median OS was significantly better for patients with LVEF>55% (13 mo); for dFLC < 400 mg/L was 7 mo (vs. 3 mo for dFLC >400 mg/L); and for those with SBP > 110 mmHg was 10 mo (vs. 5 mo in those with lower SBP). Median OS for patients achieving a CR/VGPR at day 30 was 26 mo compared to 5 mo for patients with <VGPR at that time. The median OS for patients who finally achieved CR/VGPR was 38 mo, PR 7 mo and NR was 2.6 mo (log rank p<0.0001) (Fig 1). In a multivariate model, achieving a hematological CR/VGPR (HR 5.3), LVEF <55% (HR1.5), dFLC >400 mg/L (HR 1.3) and SBP <110 mm of Hg (HR 1.5) were independent predictors of outcomes. CONCLUSIONS: The survival of stage IIIb AL remains poor but has improved compared with historical reported series. The survival of patients who achieve a CR or VGPR is over 3 years. Early achievement of ≥VGPR, as early as end of cycle 1, seems to predict for a better survival challenging the practice of treating stage III patients with "low" dose chemotherapy which often leads to slower clonal responses. This study confirms that, even in this advanced group of patients, a rapid hematological response will translate into improved outcomes. Since most non-responders to the first 1 or 2 cycles succumb to progressive disease, prospective studies are urgently needed to design rapidly effective well tolerated regimes possibly with combination of anti-amyloid therapy. Disclosures No relevant conflicts of interest to declare.

scholarly journals Comparison of Different Techniques to Identify Cardiac Involvement in Immunoglobulin Light Chain Amyloidosis

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 3182-3182
Author(s):  
Mohammed A Aljama ◽  
M Hasib Sidiqi ◽  
Angela Dispenzieri ◽  
Morie A. Gertz ◽  
Martha Q. Lacy ◽  
...  
Keyword(s):  
Strain Rate ◽  
Board Of Directors ◽  
Light Chain ◽  
Mayo Clinic ◽  
Endomyocardial Biopsy ◽  
Research Funding ◽  
Cardiac Amyloidosis ◽  
Cardiac Involvement ◽  
Al Amyloidosis ◽  
Advisory Committees

Abstract Background: Cardiac involvement is integral in staging and prognosis of immunoglobulin light chain (AL) amyloidosis. The N-terminal prohormone of brain natriuretic peptide (NT proBNP) is a cardiac biomarker used in screening for cardiac involvement and staging the disease. Transthoracic echocardiogram (TTE) and cardiac magnetic resonance (CMR) are the imaging modalities recommended to determine cardiac involvement and function. Methods: We conducted a retrospective review of all patients with biopsy proven systemic AL amyloidosis seen at the mayo clinic between Jan 1, 2006 and Dec 30, 2015. The aim of the study is to identify the nature of abnormalities in cardiac biomarkers and echocardiographic features in patients with AL amyloidosis and the ability of these investigations to diagnose cardiac involvement. We first identified all patients with AL amyloidosis that underwent endomyocardial biopsy for suspicion of cardiac involvement (Cohort 1). We then analyzed a cohort (Cohort 2) which consisted of patients who had serum NT proBNP and a comprehensive echocardiographic evaluation at time of diagnosis. Results: 179 patients with AL amyloidosis underwent endomyocardial biopsy (Cohort 1) of whom 173 had evidence of amyloid deposition. In this cohort, 159 patients had NT proBNP performed at the time of the procedure. The NT proBNP was elevated (>300) in all 159 patients with a median NT proBNP of 4917 (range 355-69541). The median left ventricular ejection fraction (LVEF), interventricular septal (IVS) thickness and strain rate were 54 (range 10-77), 15 (range 8-30) and -9 (range -21 to 0) respectively. CMR findings were consistent or suggestive of light chain amyloidosis in 38/42 patients, yielding a sensitivity of 90 percent. The LVEF, IVS thickness and strain rate were abnormal in 89/168 (53%), 102/64 (61%) and 92/95 (97%) respectively. 95 patients with biopsy proven cardiac amyloidosis had complete echocardiogram data available on LVEF, IVS thickness and strain rate, with 97% (n=92) presenting with an abnormality in at least one of these variables . CMR findings were consistent or suggestive of light chain amyloidosis in 38/42 patients, yielding a sensitivity of 90 percent. Patients with a normal NT proBNP and normal echocardiogram were considered disease free (true negative), based on our initial analysis of these investigations in Cohort 1. Cohort 2 consisted of 342 consecutive patients. The median NT pro BNP was 1878 (25-48214). The median LVEF, IVS thickness and strain rate were 63 (22-90), 14 (6-25) and -13 (-25 to -3) respectively. 259 (76%) patients had a positive NT proBNP (above 300), of whom 237 (92%) had an abnormality detected on TTE. 83 patients had a negative NT proBNP, of whom 27 (33%) had an abnormality in either LVEF, IVS thickness or strain rate. 19 of these 27 patients had a borderline reduced strain rate between -17 and -18, whilst the remaining 8 patients had a strain between -14 and -15. Only 6/27 patients were considered to have possible early cardiac involvement and none have any other diagnostic or classical features of amyloidosis on TTE. Conclusion: The combination of NT proBNP and comprehensive echocardiographic evaluation provides substantial information to diagnose cardiac amyloidosis in a significant portion of patients negating the need for endomyocardial biopsy. A negative NT proBNP rules out clinically meaningful cardiac involvement and may obviate the routine use of TTE in patients with a low clinical suspicion of cardiac amyloidosis. Disclosures Dispenzieri: Celgene, Takeda, Prothena, Jannsen, Pfizer, Alnylam, GSK: Research Funding. Gertz:Research to Practice: Consultancy; Physicians Education Resource: Consultancy; Ionis: Honoraria; celgene: Consultancy; spectrum: Consultancy, Honoraria; Teva: Consultancy; Amgen: Consultancy; Medscape: Consultancy; janssen: Consultancy; Alnylam: Honoraria; Abbvie: Consultancy; annexon: Consultancy; Apellis: Consultancy; Prothena: Honoraria. Lacy:Celgene: Research Funding. Dingli:Millennium Takeda: Research Funding; Millennium Takeda: Research Funding; Alexion Pharmaceuticals, Inc.: Other: Participates in the International PNH Registry (for Mayo Clinic, Rochester) for Alexion Pharmaceuticals, Inc.; Alexion Pharmaceuticals, Inc.: Other: Participates in the International PNH Registry (for Mayo Clinic, Rochester) for Alexion Pharmaceuticals, Inc.. Kapoor:Takeda: Research Funding; Celgene: Research Funding. Kumar:AbbVie: Membership on an entity's Board of Directors or advisory committees, Research Funding; Novartis: Research Funding; Roche: Research Funding; Celgene: Membership on an entity's Board of Directors or advisory committees, Research Funding; Oncopeptides: Membership on an entity's Board of Directors or advisory committees; Celgene: Membership on an entity's Board of Directors or advisory committees, Research Funding; KITE: Membership on an entity's Board of Directors or advisory committees, Research Funding; Takeda: Membership on an entity's Board of Directors or advisory committees; Janssen: Membership on an entity's Board of Directors or advisory committees, Research Funding; Janssen: Membership on an entity's Board of Directors or advisory committees, Research Funding; AbbVie: Membership on an entity's Board of Directors or advisory committees, Research Funding; KITE: Membership on an entity's Board of Directors or advisory committees, Research Funding; Merck: Membership on an entity's Board of Directors or advisory committees, Research Funding.

scholarly journals Quantitative assessment of left ventricular longitudinal function and myocardial deformation in Duchenne muscular dystrophy patients

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Roman Panovský ◽  
Martin Pešl ◽  
Jan Máchal ◽  
Tomáš Holeček ◽  
Věra Feitová ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Cardiac Involvement ◽  
Systolic Function ◽  
Global Longitudinal Strain ◽  
Radial Strain ◽  
Strain Analysis ◽  
Left Ventricular ◽  
Muscle Impairment ◽  
Lv Systolic Function

Abstract Background Duchenne muscular dystrophy (DMD) manifests in males mainly by skeletal muscle impairment, but also by cardiac dysfunction. The assessment of the early phases of cardiac involvement using echocardiography is often very difficult to perform in these patients. The aim of the study was to use cardiac magnetic resonance (CMR) strain analysis and mitral annular plane systolic excursion (MAPSE) in the detection of early left ventricular (LV) dysfunction in DMD patients. Methods and results In total, 51 male DMD patients and 18 matched controls were examined by CMR. MAPSE measurement and functional analysis using feature tracking (FT) were performed. Three groups of patients were evaluated: A/ patients with LGE and LV EF < 50% (n = 8), B/ patients with LGE and LVEF ≥ 50% (n = 13), and C/ patients without LGE and LVEF ≥ 50% (n = 30). MAPSE and global LV strains of the 3 DMD groups were compared to controls (n = 18). Groups A and B had significantly reduced values of MAPSE, global longitudinal strain (GLS), global circumferential strain (GCS), and global radial strain (GRS) in comparison to controls (p < 0.05). The values of MAPSE (11.6 ± 1.9 v 13.7 ± 2.7 mm) and GCS (− 26.2 ± 4.2 v − 30.0 ± 5.1%) were significantly reduced in group C compared to the controls (p < 0.05). Conclusion DMD patients had decreased LV systolic function measured by MAPSE and global LV strain even in the case of normal LV EF and the absence of LGE. FT and MAPSE measurement provide sensitive assessment of early cardiac involvement in DMD patients.

Sex difference in left ventricular global longitudinal strain in patients with systemic sclerosis: association with outcomes

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
T Gegenava ◽  
N Leeuwen ◽  
S Wijngaarden ◽  
J Vries-Bouwstra ◽  
D Cassani ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Longitudinal Strain ◽  
Cardiac Involvement ◽  
Global Longitudinal Strain ◽  
Left Ventricular ◽  
Chi Square ◽  
Female Patients ◽  
All Cause Mortality ◽  
Echocardiographic Parameters ◽  
Males And Females

Abstract Background Cardiac involvement is an important cause of hospitalization and mortality in patients with systemic sclerosis (SSc). Advanced echocardiographic measures such as global longitudinal strain (GLS) have already demonstrated to help identifying cardiac involvement and improve risk-stratification in these patients. However, possible sex differences in echocardiographic parameters including GLS have not been explored so far. Purpose To compare standard and advanced (GLS) echocardiographic parameters between male and female patients with SSc and evaluate their association with cardiovascular outcomes. Methods A total of 408 patients (345 females, 54±14 years old and 63 males 51±13 years old) were included in the study. The study endpoint was all-cause mortality combined with hospitalisations for heart failure, myocardial infarction, coronary interventions, device implantations, arrhythmias, cerebral infarction and peripheral ischemic disease. Results Males and females were comparable in terms of cardiovascular risk-factors and comorbidities but showed differences in terms of disease characteristics: greater modified rodnan skin score and higher creatine phosphokinase was observed in males as compared to females, although high NT-proBNP and deteriorated glomerular filtration rate was more prevalent in females. By standard echocardiography, male SSc patients were characterised by greater left ventricular (LV) volumes, but no difference was observed in LV ejection fraction. By advanced echocardiographic analysis, LV GLS was more preserved in female patients (−21% (IQR: −22% to −20%) as compared to males (−20% (IQR −21% to −19%), p&lt;0.001. After median follow-up of 39 months (IQR: 22–66), the combined endpoint occurred in 84 patients, males were affected significantly more frequently as compared to females (20 (32%) vs. 64 (19%), p=0.017). Kaplan-Meier survival analysis showed that impaired LV GLS (based on median value −20%) was associated with higher cumulative rates of all-cause mortality both in males and females with SSc (females: Chi-Square = 80.307 Log Rank &lt;0.001; males: Chi-Square = 4.493 Log Rank = 0.034) (Fig. 1). In univariate cox regression analyses, LV GLS was also significantly associated with the endpoint both in males and females (in males HR: 1.291, 95% CI: 1.033–1.612, p=0.025, in females HR: 1.386, 95% CI: 1.290–1.491, p&lt;0.001). Conclusions Our study shows that among patients with SSc, LV GLS is more impaired in males as compared to females but in both groups is associated with higher prevalence of death and cardiovascular hospitalization. Funding Acknowledgement Type of funding source: None

P2733Prognostic value of cardiac dysautonomia in AL amyloidosis

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Nicol ◽  
A Cescau ◽  
M Baudet ◽  
S Harel ◽  
B Royer ◽  
...  
Keyword(s):  
Mayo Clinic ◽  
Cardiac Involvement ◽  
Nyha Class ◽  
Adverse Outcomes ◽  
Blood Levels ◽  
Al Amyloidosis ◽  
Class Iii ◽  
Cardiac Denervation ◽  
A Prospective Study

Abstract Introduction Cardiac involvement is the major prognostic factor in patients with light chain amyloidosis (AL). Cardiac dysautonomia can occur early in amyloidosis and can be assess by Iodine-123-metaiodobenzylguanidine (123I-MIBG) scintigraphy. Its prognostic value has been shown in TTR amyloidosis but is unknown in AL amyloidosis. We aimed to evaluate the prognosis impact of cardiac dysautonomia in patients with AL amyloidosis. Methods We carried out a prospective study in consecutive patients with biopsy-proven AL amyloidosis. All patients underwent clinical examination, EKG, echocardiography, cardiac MRI and biological tests. The 2012 Mayo clinic prognostic classification was calculated by using blood levels of NT-proBNP, cardiac T troponin and the differential of free light chains as recommended. The sympathetic cardiac innervation was assessed by using 123I-MIBGscintigraphy and measurement of the heart-to-mediastinum uptake ratio (late H/M) in the anterior view of the chest. A cardiac denervation was defined by late H/M <1.8 4h after injection of 3 MBq/kg of 123I-MIBG. The primary end-point was all-cause mortality during follow-up. Results Fifty consecutive patients with AL amyloidosis were included. The median age was 68 years old [58–73]. By using both echocardiography and MRI, cardiac involvement was diagnosed in 33 patients (66%) and thirteen of these patients were NYHA class III or IV. By using Mayo clinic classification, patients were I, II, III and IV classes in 9 (18%), 14 (28%), 16 (32%) and 11 (22%) cases respectively. According to echocardiographic data, the median wall thickness of left ventricle was 13 mm [12–15]. The late H/M was 1.51 [1.33–1.67]. Cardiac denervation was found in 44 patients (88%). The 6 patients (12%) with a normal late H/M had no cardiac amyloidosis involvement. During a median follow-up of 24 months, 9 patients (18%) died. The area under the ROC curve of late H/M for predicting death was 0.74 (CI 95% 0.58–0.86). According to this curve, the best threshold was 1.44 and 7 of the 9 patients who died had late H/M ≤1.44. The figure shows the 2 year-survival according to late H/M. Late H/M ≤1.44 predicted all-cause death irrespective of the Mayo clinic classification: HR 8.0 (CI 95% 2.1–63) after adjustment on the Mayo clinic score (p=0.005). In addition, unplanned hospitalization for heart failure occurred in 8 patients with late H/M ≤1.44 versus 3 patients with late H/M >1.44 (p=0.03). Survival according to late H/M Conclusion Late H/M ≤1.44 is predictive of adverse outcomes in patients with AL amyloidosis, independently of the Mayo Clinic prognostic classification.

Abstract 15734: Left Atrial Reservoir Strain as a Novel Prognostic Marker in Biopsy-proven Light-chain Amyloidosis

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Peter Huntjens ◽  
Kathleen Zhang ◽  
Yuko Soyama ◽  
Maria Karmpalioti ◽  
Daniel Lenihan ◽  
...  
Keyword(s):  
Light Chain ◽  
Left Atrial ◽  
Prognostic Value ◽  
Cardiac Amyloidosis ◽  
Longitudinal Strain ◽  
Global Longitudinal Strain ◽  
Strain Imaging ◽  
Left Ventricular ◽  
Lv Function ◽  
Al Amyloidosis

Introduction: Light chain cardiac amyloidosis (AL) has a variable but usually poor prognosis. Left ventricular (LV) function measures including LV strain imaging for global longitudinal strain (GLS) have shown clinically prognostic value in AL. However, the utility of novel left atrial (LA) strain imaging and its associations with LV disease remains unclear. Hypothesis: LA strain is of additive prognostic value to GLS in AL. Methods: We included 99 consecutive patients with AL. Cardiac amyloidosis either confirmed by endocardial biopsy (25%) or by non-cardiac tissue biopsy and imaging data supportive of cardiac amyloidosis. Peak LA reservoir strain was calculated as an average of peak longitudinal strain from apical 2- and 4-chamber views. GLS and apical sparing ratio were assessed using the 3 standard apical views. All-cause mortality was tracked over a median of 5 years. Results: Echocardiographic GLS and peak longitudinal LA strain were feasible in 96 (97%) and 86 (87%) of patients, respectively. There were 48 AL patients who died during follow-up. Patients with low GLS (GLS < median; 10.3% absolute values) had worse prognosis than patients with high GLS group (p<0.001). Although peak longitudinal LA strain was correlated with GLS (R=0.65 p<0.001), peak longitudinal LA strain had additive prognostic value. AL patients with low GLS and low Peak LA strain (<13.4%) had a 8.3-fold increase in mortality risk in comparison to patients with high GLS (95% confidence interval: 3.84-18.03; p<0.001). Multivariable analysis showed peak longitudinal LA strain was significantly and independently associated with survival after adjusting for clinical and echocardiographic covariates (p<0.01). Conclusions: Peak longitudinal LA strain was additive to LV GLS in predicting prognosis in patients with biopsy confirmed AL amyloidosis. LA strain imaging has potential clinical utility in patients with AL cardiac amyloidosis.

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